A case of rhabdoid tumor of kidney, in a 2 month-old male infant, is presented. The first symptom was macroscopic hematuria. CT scan of the abdomen revealed a tumor to the left kidney and the infant underwent a left nephrectomy. The histological diagnosis was based on the characteristic "rhabdoid" appearance of the neoplastic cells. Rhabdoid tumor is considered to be one of the most malignant tumors of the early life (<2 years), comprasing 2% of childhood renal cancer. This tumor is characterized by early metastases and a poor response to therapy. In our case the patient was treated with five circles of chemotherapy, with CARBO, IFOS and ETOP. Because of no response, two circles with topotekane were added, without any results. The infant died 5 months after the diagnosis.